About: Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease     Goto   Sponge   NotDistinct   Permalink

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Description
  • NADH:ubichinon oxidoreduktáza či komplex I mitochondriálního dýchacího řetězce je složitý enzym s životně důležitou rolí v energetickém metabolismu. Dříve jsme vyvinuli hlístový model komplexu I spojený s mitochondriální dysfunkcí, jejíž rysy jsou charakteristické pro mitochondriální onemocnění jako je mléčná acidóza a snížené dýchání. Exprimovali jsme gen NDI1 ze Saccharomyces cerevisiae, v druhu Caenorhabditis elegans s poškozeným komplexem I. (cs)
  • The NADH:ubiquinone oxidoreductase or complex I of the mitochondrial respiratory chain is an intricate enzyme with a vital role in energy metabolism. We have previously developed a nematode model of complex I-associated mitochondrial dysfunction that features hallmark characteristics of mitochondrial disease, such as lactic acidosis and decreased respiration. We have expressed the Saccharomyces cerevisiae NDI1 gene in a strain of Caenorhabditis elegans with an impaired complex I. Expression of Ndi1p produces marked improvements in animal fitness and reproduction, increases respiration rates and restores mitochondrial membrane potential to wild type levels. Ndi1p functionally integrates into the nematode respiratory chain and mitigates the deleterious effects of a complex I deficit. However, we have also shown that Ndi1p cannot substitute for the absence of complex I. Nevertheless, the yeast Ndi1p should be considered as a candidate for gene therapy in human diseases involving complex I.
  • The NADH:ubiquinone oxidoreductase or complex I of the mitochondrial respiratory chain is an intricate enzyme with a vital role in energy metabolism. We have previously developed a nematode model of complex I-associated mitochondrial dysfunction that features hallmark characteristics of mitochondrial disease, such as lactic acidosis and decreased respiration. We have expressed the Saccharomyces cerevisiae NDI1 gene in a strain of Caenorhabditis elegans with an impaired complex I. Expression of Ndi1p produces marked improvements in animal fitness and reproduction, increases respiration rates and restores mitochondrial membrane potential to wild type levels. Ndi1p functionally integrates into the nematode respiratory chain and mitigates the deleterious effects of a complex I deficit. However, we have also shown that Ndi1p cannot substitute for the absence of complex I. Nevertheless, the yeast Ndi1p should be considered as a candidate for gene therapy in human diseases involving complex I. (en)
Title
  • Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease
  • Exprese proteinu Ndi1, alternativní NADH:ubichinon oxidoreduktázy, zvyšuje mitochondriální membránový potenciál v C. elegans, modelového organismu pro mitochondriální onemocnění (cs)
  • Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease (en)
skos:prefLabel
  • Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease
  • Exprese proteinu Ndi1, alternativní NADH:ubichinon oxidoreduktázy, zvyšuje mitochondriální membránový potenciál v C. elegans, modelového organismu pro mitochondriální onemocnění (cs)
  • Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease (en)
skos:notation
  • RIV/00216208:11320/07:00004673!RIV08-MSM-11320___
http://linked.open.../vavai/riv/strany
  • 1157;1163
http://linked.open...avai/riv/aktivita
http://linked.open...avai/riv/aktivity
  • Z(MSM0021620835)
http://linked.open...iv/cisloPeriodika
  • 9
http://linked.open...vai/riv/dodaniDat
http://linked.open...aciTvurceVysledku
http://linked.open.../riv/druhVysledku
http://linked.open...iv/duvernostUdaju
http://linked.open...titaPredkladatele
http://linked.open...dnocenehoVysledku
  • 421417
http://linked.open...ai/riv/idVysledku
  • RIV/00216208:11320/07:00004673
http://linked.open...riv/jazykVysledku
http://linked.open.../riv/klicovaSlova
  • Expression; Ndi1p; alternative; ubiquinone; oxidoreductase; increases; mitochondrial; membrane; potential; elegans; model; mitochondrial; disease (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...odStatuVydavatele
  • NL - Nizozemsko
http://linked.open...ontrolniKodProRIV
  • [2A97BE825364]
http://linked.open...i/riv/nazevZdroje
  • Biochimica et Biophysica Acta - Bioenergetics
http://linked.open...in/vavai/riv/obor
http://linked.open...ichTvurcuVysledku
http://linked.open...cetTvurcuVysledku
http://linked.open...UplatneniVysledku
http://linked.open...v/svazekPeriodika
  • 1767
http://linked.open...iv/tvurceVysledku
  • Gášková, Dana
http://linked.open...n/vavai/riv/zamer
issn
  • 0005-2728
number of pages
http://localhost/t...ganizacniJednotka
  • 11320
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